Fetal dextro-transposition of the great arteries (d-TGA) represents a severe cyanotic congenital heart defect. Without effective intracardiac communication, affected neonates face life-threatening hypoxemia and acidosis shortly after birth. Recent investigations have unveiled an additional dimension: d-TGA patients experience compromised cerebral development and inadequate oxygen delivery beginning in utero, subsequently manifesting as executive dysfunction and other neurodevelopmental challenges throughout childhood and adolescence—obstacles that now constitute the primary barriers to favorable long-term outcomes. Advances in prenatal ultrasonographic diagnosis, artificial intelligence-assisted technologies, and integrated multidisciplinary perinatal management have substantially elevated survival rates in recent years. Nevertheless, persistent clinical challenges remain: suboptimal prenatal detection rates, incomplete genetic counseling frameworks, insufficiently delineated perinatal treatment pathways, and delayed interventions for neurodevelopmental impairments. This narrative review integrates current knowledge regarding fetal d-TGA, endeavoring to provide actionable insights for clinical diagnosis, therapeutic strategies, and comprehensive management.